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- Gastrointestinal Stromal Tumors Treatment (PDQ®) - NCI
Most GISTs are sporadic, but there are rare familial forms associated with neurofibromatosis type 1 (NF1) or heritable variants in KIT and SDH [2, 3] GISTs rarely affect children and young adults (<1% of cases), with a median age of 15 years
- Recent Advances in Succinate Dehydrogenase Deficient Gastrointestinal . . .
Most GIST are caused by mutations in KIT or PDGFRA tyrosine kinases, and these tumors are generally treated with TKIs, including imatinib, to which SDH-Def GIST are generally resistant [3, 4] SDH-Def GIST make up the majority (~15%) of adult GIST that lack targetable KIT and PDGFRA mutations and nearly all pediatric GIST [5, 6]
- Advances in therapy of succinate dehydrogenase (SDH)-deficient . . .
Wang JH, Lasota J, Miettinen M Succinate Dehydrogenase Subunit B (SDHB) Is Expressed in Neurofibromatosis 1-Associated Gastrointestinal Stromal Tumors (Gists): Implications for the SDHB Expression Based Classification of Gists
- About Mutations in the SDHB Gene - Memorial Sloan Kettering Cancer Center
Some examples of less common cancers linked to SDHB are: GISTs (gastrointestinal stromal tumors) Kidney cancer Neuroblastoma (NOOR-oh-blas-TOH-muh) Neuroblastoma is a rare pediatric cancer that develops from an early form of nerve cells called neuroblasts
- Recent advances in the systemic treatment of gastrointestinal stromal . . .
Similar to imatinib, sunitinib has remained the standard of care in the second-line treatment of advanced GISTs since 2006; however, the recent randomized phase III trial, INTRIGUE, demonstrated the value of ripretinib, a switch-control TKI active against a broad spectrum of KIT and PDGFRA mutations, in the same setting
- Management of advanced and metastatic gastrointestinal stromal tumors
INTRODUCTION Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms affecting the gastrointestinal (GI) tract In approximately 90 percent of GIST cases, an activating mutation in KIT drives tumorigenesis However, some KIT wild-type GISTs have activating mutations in a related receptor tyrosine kinase, platelet-derived growth factor receptor alpha (PDGFRA) Treatment of GISTs was
- The GIST of Advances in Treatment of Advanced Gastrointestinal Stromal . . .
All currently approved agents for the treatment of GIST are based on orally available tyrosine kinase inhibitors targeting KIT and PDGFRA oncogenic activation Although first-line imatinib achieves remarkable prolonged disease control, the benefit of subsequent lines of treatment is more modest
- SDH-Deficient Gastrointestinal Stromal Tumor (GIST) - NCI
SDH-deficient gastrointestinal stromal tumor, or SDH-deficient GIST, is a type of digestive tract cancer GIST is a fairly common type of cancer, but SDH-deficient GIST makes up only about 6% of GIST diagnoses Learn more about how this tumor an run in families, the treatment, and the prognosis
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